Retinoblastoma is a disease in which malignant (cancer) cells form in the tissues of the retina.
The retina is the nerve tissue that lines the inside of the back of the eye. The retina senses light and sends images to the brain by way of the optic nerve.
Anatomy of the eye, showing the outside and inside of the eye including the sclera, cornea, iris, ciliary body, choroid, retina, vitreous humor, and optic nerve. The vitreous humor is a gel that fills the center of the eye.
Although retinoblastoma may occur at any age, it usually occurs in children younger than 5 years, most often younger than 2 years. The tumor may be in one eye or in both eyes. Retinoblastoma rarely spreads from the eye to nearby tissue or other parts of the body.
Retinoblastoma occurs in heritable and nonheritable forms.
A child is thought to have the heritable form of retinoblastoma when one of the following is true:
- There is a family history of retinoblastoma.
- There is more than one tumor in the eye.
- There is a certain change in the retinoblastomagene.
Nonheritable retinoblastoma is retinoblastoma that is not the heritable form.
After diagnosis and treatment in a child with heritable retinoblastoma, new tumors may continue to form for a few years. Regular eye exams to check for new tumors are usually done every 2 to 4 months for at least 28 months.
The parents, brothers, and sisters of a child with retinoblastoma need to have their eyes checked for retinoblastoma.
The parents of a child with retinoblastoma should have an eye exam by an ophthalmologist (a doctor with special training in diseases of the eye).
The brother or sister of a child with retinoblastoma also should have regular eye exams by an ophthalmologist until age 3 to 5 years, unless it is known that the brother or sister does not have the retinoblastoma gene change. Children who have a close family member with retinoblastoma should have regular eye exams beginning early in life to check for retinoblastoma. Early diagnosis of retinoblastoma may mean the child will need less intense treatment.